Anaplastic Large Cell Lymphoma of the Breast: Incidence, Patterns of Care, and Outcome in the US Population

Background:

Anaplastic large cell lymphoma (ALCL) of the breast is a recently described clinicopathologic entity, almost always associated with breast implants, and does not overexpress the anaplastic lymphoma kinase (ALK) protein. Using the two largest US cancer databases, we describe its incidence, patterns of care, and clinical outcome.

Methods:

Incidence rate was calculated using 2000-2011 data from the Surveillance, Epidemiology, and End Results (SEER) Program and age-adjusted to the US 2000 standard population. For patient level data, we used the National Cancer Data Base (NCDB) Participant User File. We included all adult patients diagnosed from 1998-2011 with ALCL (ICD-O-3: 99714) who had breast as the primary site.

Results:

The incidence of breast ALCL was 0.001 per million. We identified 61 cases from NCDB. All of the patients were females and they were predominantly White (88.5%). The median age at diagnosis was 64 years (range, 18-90). The stage distribution was I (45.9%), II (21.3%), III (0%), IV (9.8%), and unknown (21.3%). The pattern of initial treatment was surgery alone (13.1%), chemotherapy alone (32.7%), chemotherapy and radiation (19.7%), surgery and chemotherapy (4.9%), surgery and radiation (4.9%), and tri-modality treatment (13.1%). In 7 patients (11.5%), no treatment was given. Two patients (1.6%) received stem cell transplant (1 autologous, 1 allogeneic) as part of initial treatment, one after chemotherapy and another one after surgery and chemotherapy. Overall survival data was available for patients diagnosed in 1998-2006 (29 patients). The median overall survival was 149.8 months (range, 2.6-157.9).

Conclusions:

ALCL of the breast is very rare. It presents mainly as early stage disease with very good prognosis. The patterns of care varied, although most patients received chemotherapy as part of the initial treatment.